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J Korean Acad Pediatr Dent. 2010;37(1): 124-129.
SECKEL SYNDROME : CASE REPORTS
Chu-Sung Kim, Jae-Gon Kim, Byeong-Ju Baik, Yeon-Mi Yang, Jin-Woo Jeong
Department of Pediatric dentistry and Institute of Oral Bioscience, School of Dentistry, Chonbuk National University
Corresponding Author: Jae-Gon Kim ,Tel: 063-250-2128 /, Email: pedodent@chonbuk.ac.kr
Received: April 6, 2009;  Accepted: September 15, 2009.
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ABSTRACT
Seckel syndrome is an autosomal recessive disorder characterized by intrauterine growth retardation and post-natally by dwarfism, severe microcephaly, bird-headed profile with receding chin, prominent nose, joint defects, clubfoot, sparse hair, malformation of genitourinary tract and rectum, mental retardation and hematological disorders. There is also a reduction in the number of blood cells. Dental anomalies of Seckel syndrome are crowded teeth with malocclusion, enamel hypoplasia, absence of some teeth and taurodontism. This report described the oral and maxillofacial manifestations of children associated with Seckel syndrome. Children with Seckel syndrome have several dental and skeletal irregularities. The purpose of this study was to report the dental and medical characteristics of the patient and review the literatures of Seckel syndrome.
Keywords: Seckel syndrome | Bird-headed profile | Microcephaly | Dwarfism
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